Digestive hemorrhage due to stromal tumor located in the jejunum in a patient with neurofibromatosis

Rafael Cerdán Pascual; Joaquín Bernal Jaulín; Sonia Cantín Blázquez; José Ignacio Barranco Domínguez; Sofía Borlán Ansón; Alejandro Serrablo Requejo; Jesús María Esarte Muniain

2007, Number 1

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Cir Gen 2007; 29 (1)

Digestive hemorrhage due to stromal tumor located in the jejunum in a patient with neurofibromatosis

Cerdán PR, Bernal JJ, Cantín BS, Barranco DJI, Borlán AS, Serrablo RA, Esarte MJM

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Language: Spanish
References: 19
Page: 54-57
PDF size: 90.80 Kb.

ABSTRACT

Objective: To report the case of a female patient with neurofibromatosis and gastrointestinal hemorrhage induced by jejunal stromal tumor.
Setting: Third level health care hospital.
Case description: Gastrointestinal stromal tumors (GIST) are originated in the mesenquimal tissue of the gastrointestinal wall. They are usually located in the stomach, and the jejunum is the second most frequent location. We report a case of a 57-year-old woman, with neurofibromatosis type 1 and admitted to the hospital because of an acute gastrointestinal bleeding. Laparotomy revealed a proximal jejunal tumor that was classified as GIST. We analyze the ultrastructural characteristics of these tumors, their forms of presentation, and their diagnostic difficulties when lesions are located in the small bowel.
Conclusion: Association between GIST and neurofibromatosis is an inusual finding, frequently producing symptoms that require surgical treatment.

REFERENCES

Tubía LJI, Sánchez PA, Partearroyo SN, Galarraga II, López HAE, Palomar LM, et al. Tumor estromal CD 34+ de localización en yeyuno. Presentación de un caso que se inicia como hemorragia digestiva. Cir Esp 2000; 67: 100-2.
Mirón PB, Becerra MA, Romera LAL, Rueda LAI, Huertas PF, Pérez CB, et al. Tumores de la estroma gastrointestinal y neurofibromatosis tipo 1. Cir Esp 2003; 74: 171-3.
Martínez OJL, Esmer SDD, Suárez MR, Ruiz RR, Blanco BR. Neurofibromatosis gastrointestinal. Una causa poco frecuente de cirugía abdominal. Rev Gastroenterol Mex 2000; 65: 34-6.
De Silva CM, Reid R. Gastrointestinal stromal tumors (GIST): c-kit mutations, CD117 expression, differential diagnosis and targeted cancer therapy with Imatinib. Pathol Oncol Res 2003; 9: 13-9.
Ma CK, Amin MB, Kintanar E, Linden MD, Zarbo RJ. Immunohistologic characterization of gastrointestinal stromal tumors: A study of 82 cases compared with 11 cases of leiomyomas. Mod Pathol 1993; 6: 139-144.
Suster S. Gastrointestinal stromal tumors. Semin Diagn Pathol 1996; 13: 297-313.
Milettinen M, Virolainen M, Maarit-Sarlomo-Rikala. Gastrointestinal stromal tumors— value of CD34 antigen in their identification and separation from true leiomyomas and schwannomas. Am J Surg Pathol 1995; 19: 207-216.
Shojaku H, Futatsuya R, Seto H, Tajika S, Matsunou H. Malignant gastrointestinal stromal tumor of the small intestine: radiologic-pathologic correlation. Radiat Med 1997; 15: 189-192.
Tworek JA, Appelman HD, Singleton TP, Greenson JK. Stromal tumors of the jejunum and ileum. Mod Pathol 1997; 10: 200-209.
Dougherty MJ, Compton C, Talbert M, Wood WC. Sarcomas of the gastrointestinal tract. Separation into favorable and unfavorable prognostic groups by mitotic count. Ann Surg 1991; 214: 569-574.
Prieto NL, Pérez RJP, Rodríguez MJA, Corripio R, García SML. Leiomiosarcoma intestinal perforado. Rev Esp Enferm Dig 1998; 90: 730-731.
Lillemoe KD, Efron DT. Gastrointestinal stromal tumors. En: Cameron JL, editor. Current surgical therapy. 7th ed. St. Louis: Mosby Inc., 2001: 112-7.
Min KW. Small intestinal stromal tumors with skeinoid fibers. Clinicopathological, immunohistochemical, and ultraestructural investigations. Am J Surg Pathol 1992; 16: 145-155.
Ueyama T, Guo KJ, Hashimoto H, Daimaru Y, Enjoji M. A clinicopathologic and immunohistochemical study of gastrointestinal stromal tumors. Cancer 1992; 69: 947-955.
Zuckerman DA, Bocchini TB, Birnbaum EH. Massive hemorrhage in the lower gastrointestinal tract in adults: diagnostic imaging and intervention. AJR Am J Roentgenol 1993; 161: 703-711.
DeMatteo RP, Heinrich MC, El-Rifai WM, Demetri G. Clinical management of gastrointestinal stromal tumors: before and after STI-571. Hum Pathol 2002; 33: 466-77.
Taniguchi M, Nishida T, Hirota S, Isozaki K, Ito T, Nomura T, et al. Effect of c-kit mutation on prognosis of gastrointestinal stromal tumors. Cancer Res 1999; 59: 4297-300.
Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, et al. Diagnosis of gastrointestinal stromal tumors: A consensus approach. Hum Pathol 2002; 33: 459-65.
DeMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF. Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg 2000; 231: 51-8.